Introduction: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients.
Materials And Methods: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire.
Results: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient.
Conclusions: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors.
Type Of The Study: retrospective case series.
Level Of Evidence: IV.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jpedsurg.2018.10.060 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Structural Disruption of Cilia and Increased Cytoplasmic Tubulin in Biliary Atresia-An Exploratory Study Focusing on Early Postoperative Prognosis Following Portoenterostomy.
Biomedicines
January 2025
Faculty of Health Sciences, Health Science Investigation Center of University of Beira Interior (CICS-UBI), 6200-506 Covilhã, Portugal.
Biliary atresia (BA) is a progressive hepatobiliary disease in infants, leading to liver failure and the need for transplantation. While its etiopathogenesis remains unclear, recent studies suggest primary cilia (PC) disruption plays a role. This study investigates correlations between PC and cytoplasmic tubulin (TUBA4A) alterations with hypoxia in patients with the isolated form of BA, focusing on native liver survival.
View Article and Find Full Text PDFMapping cells through time and space with moscot.
Nature
January 2025
Institute of Computational Biology, Helmholtz Center, Munich, Germany.
Single-cell genomic technologies enable the multimodal profiling of millions of cells across temporal and spatial dimensions. However, experimental limitations hinder the comprehensive measurement of cells under native temporal dynamics and in their native spatial tissue niche. Optimal transport has emerged as a powerful tool to address these constraints and has facilitated the recovery of the original cellular context.
View Article and Find Full Text PDFPrediction of Native Liver Survival in Patients With Biliary Atresia 20 Years After the Kasai Procedure.
J Pediatr Surg
January 2025
Objective: To establish risk models for long-term native liver survival (NLS) in patients with biliary atresia.
Methods: In this retrospective study, we analyzed data from 1792 patients registered in the Japanese Biliary Atresia Registry. Using multivariate logistic regression, we created predictive models for NLS at 1, 5, 10, 15, and 20 years postoperatively.
Use of laser-ablation inductively-coupled mass spectroscopy for analysis of selenosugars bound to proteins.
Metallomics
January 2025
Department of Nutritional Sciences, University of Wisconsin, Madison, WI 53706, USA.
We previously used high pressure liquid chromatography (HPLC) coupled with Se-specific inductively coupled plasma mass spectrometry (ICP-MS) and molecule specific (ESI Orbitrap MS/MS) detection to study the increase in liver Se in turkeys and rats supplemented as selenite in high-Se (5 µg Se/g diet) and adequate-Se diets. We found that far more Se is present as selenosugar (seleno-N-acetyl galactosamine) than is present as selenocysteine (Sec) in true selenoproteins. In high-Se liver, the increase in liver Se was due to low molecular weight (LMW) selenometabolites as glutathione-, cysteine- and methyl-conjugates of the selenosugar, but also as high molecular weight (HMW) species as selenosugars decorating general proteins via mixed-disulfide bonds.
View Article and Find Full Text PDFLiver Xenotransplantation: A Path to Clinical Reality.
Transpl Int
January 2025
Division of Transplant Surgery, Department of Surgery, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, United States.
Liver xenotransplantation has emerged as a potential solution to the shortage of deceased human donor organs and is now becoming a reality due to recent developments in genetic engineering and immunosuppressive therapy. Early efforts using non-human primates and genetically modified pigs faced significant challenges such as thrombocytopenia and graft rejection. Understanding the mechanism behind those challenges and using novel genetically engineered pigs enabled researchers to overcome some of the hurdles, but more research is needed.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!